Well friends and family – I am happy to say that after several attempts made by multiple doctors, and 200+ hours of efforts on my part (research, synthesizing information, phone calls, e-mails) over the past 2 years, my insurance company has finally been convinced IVIG is a medical necessity in my case!!!

“After careful review of the information we have, the appeal request has been approved. There are extenuating circumstances such that this service is medically necessary for this patient.” – music to my ears

I’m going to keep this post basic. (Friendly hint to someone I know who always forgets: the underlined words in this post are links.)

What is it?

You may remember me trying a medication in early 2015? That’s this medication. My trial in 2015 was abruptly stopped after 2 months because the infusion company made an error in their insurance verification process, and I was never supposed to have gotten it in the first place. Whoops!

January 2015 – my first IVIG infusion ever

This was a costly mistake (that they wanted me to pay for) given that this medication costs $10,000 per month on average, however there is a silver lining. A huge part of why I am being allowed to try IVIG is because I experienced improvements while on it in 2015.

IVIG stands for intravenous immunoglobulin. The name pretty much says it all. It is a medication made up of human immunoglobulins (antibodies) – which are obtained from people donating plasma – and it is infused through an IV.

What are your hopes for this medication?

I have many hopes for this treatment, but absolutely no expectations, if that makes any sense. Let’s play best case/worst case scenario.

Best CaseIt regulates my immune system and halts the progression of my autoimmune diseases. As a result, I no longer have fevers several days a week. Due to my body no longer being under constant attack, I am not so tired all of the time. My affected systems will work better overall, because who wouldn’t work better without a bunch of immune cells trying to beat them up?

I am able to do normal activities without a major increase in symptoms. I can go to a family gathering and not be sick for a week afterward.

It reverses my nerve damage, which was caused by the autoimmune diseases. There is improvement in the disabling symptoms I experience that are attributed to nerve damage – tachycardia, blood pressure issues, inability to stand long, issues digesting food, nausea, vomiting, poor circulation, headaches, dizziness, burning and sharp nerve pain in my extremities.

Worst CaseIt does absolutely nothing. Even worse than doing nothing, it does nothing and I experience tons of side effects. You may recall I got aseptic meningitis after my first IVIG infusion in 2015. Steps have been taken to decrease the likelihood of this happening again. The infusions are being spaced out differently, and we are running the infusions extremely slow.

IVIG isn’t risk free, but it is a fairly safe treatment considering my options. People born without a functioning immune system get a much lower dose of IVIG their entire lives. Thanks to screening protocols, it is rare for transmissible diseases to be transmitted by IVIG. For someone my age with a low blood clot risk, good kidney function, and no cardiovascular risk factors, it would be incredibly unusual for a fatal complication to occur. My doctor will regularly monitor my blood work to make sure my organs are all happy campers.

Complications are not something I am afraid of or worried about. The scariest thing to me right now is the thought of being this sick the rest of my life.

Most Likely CaseNo one can answer this question. There isn’t enough research to identify why it works for some autoimmune dysautonomia patients and not for others. There is no guarantee it will reverse the nerve damage or increase my quality of life. My doctors say it will take a full year before we know with certainty if it will help or not.

That said, the “most likely case” scenario is probably somewhere between the best and worst case scenarios. In the two months I received it in 2015, the main symptom that improved were my fevers and my blood pressure issues.

Why This Medication? – It Seems Extreme

There is not much left to try. This is not a first-line treatment for dysautonomia or Sjogren’s syndrome. I have tried over 30 different treatments (medications, lifestyle modifications, supplements, programs) for my assorted conditions. There is not a single treatment for POTS I have not tried, with the exception of Desmopressin. (The utility and safety profile of desmopressin in patients like me is unclear.) My case is unusually refractory to treatment. This is likely because my autoimmune issues have gone unchecked for nearly 9 years, and since they caused nerve damage, my autoimmune issues are the root cause of my case of Dysautonomia/POTS.

When do you start?

I will be receiving infusions once every two weeks for one year. Last time, my infusions took about 8 hours each time. Some people tolerate IVIG being infused quickly, but I am not one of them (hence the aseptic meningitis).

I believe I will start sometime in the next few weeks. I would like to get one last round of blood work right before starting. Long-term, it will be interesting to see any changes – the more data points the better! One of my dreams is to write and publish a case study about myself using all the information I have collected. (I need to befriend a published researcher. Any takers?)

Where will it be done?

The infusions will be done at my house. A registered nurse will come and stay here the entire time. I believe I will get the same nurse as last time which is excellent news. She understands my conditions and doesn’t get impatient about the long infusion time.

Looking Forward

While I am grateful they finally are allowing me to try IVIG, I will not miss making phone calls to my insurance company. Most of the insurance reps were helpful and professional. However, one particular representative frustrated me quite a bit — to the point that when I type her name into my phone, it automatically corrects her name to all capital letters.

Since I will no longer be investing my extra time and energy into getting IVIG approved, I am looking forward to having more time to write here. My game plan is to post an update on how IVIG is going at least once a month.

Thanks Angela! It’s nice when hard work pays off. I was starting to think it wouldn’t this time. No matter what happens in the next year, I will consider it progress because we will finally know if it helps me or not.

I hope everything is going okay with all of the news you got recently.

Thanks hun. It’s been a roller coaster of emotions and the med changes feel like they’re going to be the death if me before I ever have an AA. But I’m trucking through it!

Next step is to have the kiddos checked for the defective gene. Also, the geneticist just sent release forms for my dad to sign in hopes there is some of my moms pathology samples still on file from her lengthy hospital stay before she passed. Since the MYLK gene defect is rare alongside EDS, they’re curious to find out if my mom carried the defect or whether it started with me.

Lol I shouldn’t laugh but I still shake my head and have to find weird amusement in the fact that I don’t carry the genes associated with VEDS but have a different gene defect that carries the same aortic risks. At least the genetic counselor knows my personality and was cracking jokes with me about dodging one bullet to catch another. 😛 No sense worrying right! But I am being proactive in regards to my kiddos incase the “what if” happens.

Now the maple syrup thing…weird! I’ll have to let you know what comes of that!

Jackie, when I saw your post, it made me incredibly happy because we have all read how much work and dedication you have put into this. All good wishes your way and praying this brings relieve to your life.

If my count is right, I was denied 13 times. I was actually going to give up if I got denied at the highest appeal level this time. Then it did – I was shocked. For this round of trying to get approved, I was at the second to highest level – next would have been state board of insurance.

I’m sorry to hear you’ve been through a similar situation with your insurance and lost. It’s sad to have to fight so hard for a medication that has the potential to change your life.

While I understand their logic, I wish there were programs to help with copayments for off label use of IVIG. The makers of IVIG have a variety of assistance programs for those using it on label.

I am so happy for you Jackie. I knew you would get through to them eventually. Too bad it took so long, but now we just look forward to the treatments helping you again and you feeling better. Love you sweetie.

Hooray! I’m so happy for you! All your hard work paid off! You are amazing & I wish all the best that the IVIG provides some relief! Thank you for sharing the good news & as always, your updates Are most appreciated!

Hi, Jackie! I take immunoglobulins every week with subcutaneous needles. I’ve been using it for 2 years now this way. I used to get the iv doses, but I had allergy problems with the iv route. If you have problems ask about subcutaneous administration. I have not had any problems with the subcutaneous route and I can do the infusion at home without having to sit in an infusion center all day.

How interesting you tolerated sub-q, but you had allergic reactions to immunoglobulin via the IV route. I had heard it’s much better in terms of side effects, but I didn’t realize it could be that much better for some people.

They’ve mentioned maybe switching to sub-q depending how IVIG goes. The only con I’ve heard of is that people with larger doses have a bump for a few days while it absorbs after injection – is that the case for you? Most people I’ve talked to say they have way fewer side effects with sub-q.

I hope this works well for you! I was just diagnosed with Dysautonomia and probable POTS. I was diagnosed with everything else first………………………I look forward to following your blog and seeing how you are doing with this new treatment. Take care.:)

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